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The aim of this study was to improve understanding of exercise medicine training needs for pediatric cardiology fellows. A survey was sent via email to all (N = 63) pediatric cardiology training program directors in the United States to evaluate the perceived exercise training needs of pediatric cardiology fellows. The survey consisted of multiple-choice responses as well as a few open-ended responses. A 60% response rate was achieved. 74% of programs did not have a pre-existing exercise core program. This type of training was felt to be important or very important in 84%. A wide variability of time allotted for exercise training exists amongst programs from 4 weeks, with 2 weeks being most common. There was no consensus on a target number of total exercise tests nor types of tests in which fellows should participate. Preferred methods in training consisted of lectures and online media. Less preferred methods of teaching methods included dedicated reading of a handbook, a dedicated rotation, or live webinars. There was general support to develop exercise training competencies as well as the associated online learning materials with a focus on competency rather than target numbers. There is a need for educational recommendations for exercise training in pediatric cardiology fellowships as well as a unified method of achieving competencies.

INTRODUCTION: Symptoms are the most common indication for ablation in children with atrioventricular nodal reentrant tachycardia (AVNRT). After the procedure, patients may continue to report palpitations. The objective of this study was to quantify the risk and duration of palpitations after pediatric slow pathway modification as well as demographic and technical associations. METHODS: This was a retrospective review of consecutive patients at a pediatric center who underwent slow pathway modification for AVNRT from 2012–2018. Patients with a prior ablation attempt or congenital heart disease were excluded. RESULTS: Palpitations were documented in 35% of patients after ablation. Neither post-ablation echo beats nor other evidence of residual dual AV nodal physiology were associated with a higher risk of post-ablation palpitations. Of the 35 patients with post-ablation palpitations, the median time to resolution of palpitations was 48 months. Acute procedural success was achieved in all 100 cases. There were two recurrences of AVNRT during long-term follow-up and one instance of ectopic atrial tachycardia (3% SVT recurrence). CONCLUSION: Palpitations after AVNRT ablation occurred in approximately one-third of cases, despite a low recurrence of true arrhythmia. Prior to ablation, patients and families should be counseled that post-ablation palpitations are common and AVNRT recurrence is rare.

Summary Objective: Twenty-four hour ambulatory electrocardiograms (“Holter” monitors) are a key diagnostic test in cardiology. Commercial electronic medical record (EMR) tools have not been designed for pediatric Holter monitor reporting and paper-based methods are inefficient. Methods: Our tertiary pediatric hospital adapted a radiology EMR tool to a cardiology workflow in order to report Holter monitor results. A retrospective review was performed at 4 time points: prior to intervention, immediately post-intervention, at 6 months and at 12 months post-intervention. The primary outcome variable was time to reporting of Holter findings. Results: Holter reports were reviewed on 527 studies (patient ages: 1 day to 42 years). The time between the date the patient returned the Holter monitor until the date the referring physician received a final report improved from 19.8 days to 1.5 days (p Conclusion: EMR systems are not typically designed for pediatric cardiology, but existing systems can be adapted, yielding important gains for patient care. In specialties like pediatric cardiology, there is insufficient volume nationally to drive development of commercial systems. This study demonstrates the general principle that creative adaptation of EMR systems can improve result reporting in pediatric cardiology and likely in other cardiology practices.Citation: Webster G, Ward K, Deal BJ, Anderson JB, Tsao S. Adaptation of Radiology Software to Improve Cardiology Results Reporting. Appl Clin Inform 2017; 8: 936–944 https://doi.org/10.4338/ACI-2017-03-RA-0051

Background Initial propranolol recommendations for infantile hemangioma published in 2013 were intended as provisional best practices to be updated as evidence-based data emerged. Methods A retrospective multicenter study was performed to evaluate utility of prolonged monitoring after first propranolol dose and escalation(s). Inclusion criteria included diagnosis of hemangioma requiring propranolol of greater than or equal to 0.3 mg/kg per dose, younger than 2 years, and heart rate monitoring for greater than or equal to 1 hour. Data collected included demographics, dose, vital signs, and adverse events. Results A total of 783 subjects met inclusion criteria; median age at initiation was 112 days. None of the 1148 episodes of prolonged monitoring warranted immediate intervention or drug discontinuation. No symptomatic bradycardia or hypotension occurred during monitoring. Mean heart rate change from baseline to 1 hour was –8.19/min (±15.54/min) and baseline to 2 hours was –9.24/min (±15.84/min). Three preterm subjects had dose adjustments because of prescriber concerns about asymptomatic vital sign changes. No significant difference existed in pretreatment heart rate or in heart rate change between individuals with later adverse events during treatment and those without. Conclusion Prolonged monitoring for initiation and escalation of oral propranolol rarely changed management and did not predict future adverse events. Few serious adverse events occurred during therapy; none were cardiovascular.

AimThe aim of the present study was to determine general paediatrician knowledge, practices, and attitudes towards electrocardiogram (ECG) screening in school athletes during pre-participation screening exam (PPSE).MethodsPaediatricians affiliated with a tertiary children’s hospital completed a survey about ECGs for PPSE.ResultsIn total, 205/498 (41%) responded; 92% of the paediatricians did not include an ECG as part of PPSE; 56% were aware of a case in which a student athlete in their own community had died of sudden unexplained death; 4% had an athlete in their practice die. Only 16% of paediatricians perform all 12 American Heart Association recommended elements of the PPSE. If any of these screening elements are abnormal, 69% obtain an ECG, 36% an echocardiogram, and 30% restrict patients from sports activity; 73% of them refer the patient to a cardiologist.ConclusionMost of the general paediatricians surveyed did not currently perform ECGs for PPSE. In addition, there was a low rate of adherence to performing the 12 screening elements recommended by the American Heart Association. They have trouble obtaining timely, accurate ECG interpretations, worry about potential unnecessary exercise restrictions, and cost-effectiveness. The practical hurdles to ECG implementation emphasise the need for a fresh look at PPSE, and not just ECG screening. Improvements in ECG performance/interpretation would be necessary for ECGs to be a useful part of PPSE.

Lifestyle changes such as exercise and dietary change are recommended first-line therapy for children with dyslipidemia, hypertension, and obesity. Although most clinicians recommend exercise, specific exercise prescriptions are not usually provided. The optimal type, duration, and intensity of activity to achieve a meaningful outcome is not definitively defined. As the pediatric population becomes more sedentary and morbidity accumulates from this lifestyle, understanding how targeted exercise prescriptions can benefit patients will be critical to effectively manage this group of patients. This review focuses on the role of exercise for the treatment of pediatric patients with dyslipidemia, hypertension, and obesity and describes additional factors that require future research to achieve desired outcomes in this at-risk population. [ Pediatr Ann . 2018;47(12):e494–e498.]

Infantile hemangiomas (IHs) are common neoplasms composed of proliferating endothelial-like cells. Despite the relative frequency of IH and the potential severity of complications, there are currently no uniform guidelines for treatment. Although propranolol has rapidly been adopted, there is significant uncertainty and divergence of opinion regarding safety monitoring, dose escalation, and its use in PHACE syndrome (PHACE = posterior fossa, hemangioma, arterial lesions, cardiac abnormalities, eye abnormalities; a cutaneous neurovascular syndrome characterized by large, segmental hemangiomas of the head and neck along with congenital anomalies of the brain, heart, eyes and/or chest wall). A consensus conference was held on December 9, 2011. The multidisciplinary team reviewed existing data on the pharmacologic properties of propranolol and all published reports pertaining to the use of propranolol in pediatric patients. Workgroups were assigned specific topics to propose protocols on the following subjects: contraindications, special populations, pretreatment evaluation, dose escalation, and monitoring. Consensus protocols were recorded during the meeting and refined after the meeting. When appropriate, protocol clarifications and revision were made and agreed upon by the group via teleconference. Because of the absence of high-quality clinical research data, evidence-based recommendations are not possible at present. However, the team agreed on a number of recommendations that arose from a review of existing evidence, including when to treat complicated IH; contraindications and pretreatment evaluation protocols; propranolol use in PHACE syndrome; formulation, target dose, and frequency of propranolol; initiation of propranolol in infants; cardiovascular monitoring; ongoing monitoring; and prevention of hypoglycemia. Where there was considerable controversy, the more conservative approach was selected. We acknowledge that the recommendations are conservative in nature and anticipate that they will be revised as more data are made available. Pediatrics 2013;131:128-140

Background The evolving operative strategy and course of 111 consecutive Fontan conversions with arrhythmia surgery and pacemaker therapy were reviewed to identify risk factors for poor outcome. Methods Since 1994, 111 patients (mean age 22.5 ± 7.9 years) underwent Fontan conversion with arrhythmia surgery. The series was divided into three time periods: (1) 1994 to 1996 (initial isthmus ablation, n=9, group I); (2) 1996 to 2003 (early modified right atrial maze and Cox-maze III, n=51, group II); and (3) 2003 to 2006 (recent modifications of the modified right atrial maze and left atrial Cox-maze III for both atrial fibrillation and left atrial reentry tachycardia, n=51, group III). Results There were one early (0.9%) and six late deaths (5.4%); six patients required cardiac transplantation (5.4%). Two late deaths occurred after transplantation. Renal failure requiring dialysis occurred in four patients (3.6%). Mean hospital stay was 13.7 ± 12.1 days. Mean cross-clamp time was 70.8 ± 41.6 minutes. Four risk factors for death or transplantation were identified: presence of a right or ambiguous ventricle, preoperative protein-losing enteropathy, preoperative moderate-to-severe atrioventricular valve regurgitation, and long (>239 minutes) cardiopulmonary bypass time. In intergroup comparisons (groups I and II versus group III), three trends were noted: increased incidence of concomitant surgical repairs ( p = 0.03), older patients ( p = 0.01), and increased incidence of left atrial reentry tachycardia and atrial fibrillation ( p = 0.04). Late recurrence of atrial tachycardia ensued in 15 of 111 (13.5%); 8 of 51 in group II (15.7%) and 4 of 51 in group III (7.8) ( p = 0.3). Conclusions Fontan conversion with arrhythmia surgery is safe and efficacious. Based on improved results and evolving surgical techniques, selection criteria can be more clearly defined.

Cardiopulmonary resuscitation (CPR) has been a mainstay of immediate response to acute cardiorespiratory failure since it was first introduced by Kouwenhoven et al. in 1960. The goal of CPR is to maximize coronary and cerebral blood flow and to restore spontaneous circulation. To these ends, the principles are largely unchanged from earlier descriptions, although the understanding of the physiology underlying successful resuscitation continues to grow. In this brief review, we plan to review the physiologic basis of CPR, describe some newer concepts of circulatory adjuncts, detail the rationale for pharmacotherapy, and discuss the outcomes for patients who require CPR.

The principles of Fontan conversion with arrhythmia surgery are to restore the cardiac anatomy by converting the original atriopulmonary connection to a total cavopulmonary artery extracardiac connection and treat the underlying atrial arrhythmias. Successful outcomes of this procedure are dependent on a thorough understanding of several factors: the patient's fundamental diagnosis of single-ventricle anatomy, the resultant cardiac configuration from the original atriopulmonary Fontan connection, right atrial dilatation that leads to atrial flutter or fibrillation, and associated congenital cardiac anomalies. The purpose of this article is to present some of the more challenging anatomic and electrophysiologic problems we have encountered with Fontan conversion and arrhythmia surgery and the innovative solutions we have used to treat them. The cases reviewed herein include: takedown of a Bjork-Fontan modification, right ventricular hypertension and tricuspid regurgitation after atriopulmonary Fontan for pulmonary atresia and intact ventricular septum, takedown of atrioventricular valve isolation patch for right-sided maze procedure, resultant hemodynamic considerations leading to intraoperative pulmonary vein stenosis after Fontan conversion, unwanted inferior vena cava retraction during the extracardiac connection, right atrial cannulation in the presence of a right atrial clot, distended left superior vena cava causing left pulmonary vein stenosis, dropped atrial septum, and the modified right-sided maze procedure for various single-ventricle pathology. Since 1994 we have performed Fontan conversion with arrhythmia surgery on 109 patients with a 0.9% mortality rate. We attribute our program's success in no small measure to the strong collaborative efforts of the cardiothoracic surgery and cardiology teams.

The evolving operative strategy and course of 111 consecutive Fontan conversions with arrhythmia surgery and pacemaker therapy were reviewed to identify risk factors for poor outcome.Since 1994, 111 patients (mean age 22.5 +/- 7.9 years) underwent Fontan conversion with arrhythmia surgery. The series was divided into three time periods: (1) 1994 to 1996 (initial isthmus ablation, n = 9, group I); (2) 1996 to 2003 (early modified right atrial maze and Cox-maze III, n = 51, group II); and (3) 2003 to 2006 (recent modifications of the modified right atrial maze and left atrial Cox-maze III for both atrial fibrillation and left atrial reentry tachycardia, n = 51, group III).There were one early (0.9%) and six late deaths (5.4%); six patients required cardiac transplantation (5.4%). Two late deaths occurred after transplantation. Renal failure requiring dialysis occurred in four patients (3.6%). Mean hospital stay was 13.7 +/- 12.1 days. Mean cross-clamp time was 70.8 +/- 41.6 minutes. Four risk factors for death or transplantation were identified: presence of a right or ambiguous ventricle, preoperative protein-losing enteropathy, preoperative moderate-to-severe atrioventricular valve regurgitation, and long (239 minutes) cardiopulmonary bypass time. In intergroup comparisons (groups I and II versus group III), three trends were noted: increased incidence of concomitant surgical repairs (p = 0.03), older patients (p = 0.01), and increased incidence of left atrial reentry tachycardia and atrial fibrillation (p = 0.04). Late recurrence of atrial tachycardia ensued in 15 of 111 (13.5%); 8 of 51 in group II (15.7%) and 4 of 51 in group III (7.8) (p = 0.3).Fontan conversion with arrhythmia surgery is safe and efficacious. Based on improved results and evolving surgical techniques, selection criteria can be more clearly defined.

Objective To examine heart rate recovery (HRR) as an indicator of autonomic nervous system dysfunction after maximal exercise testing in children and young adults with sickle cell anemia (SCA). Study design Recovery phase heart rate (HR) in the first 5 minutes after maximal exercise testing in 60 subjects with SCA and 30 matched controls without SCA was assessed. The difference between peak HR and HR at both 1-minute (ΔHR 1min ) and 2-minutes recovery was our primary outcome. Results Compared with controls, subjects with SCA demonstrated significantly smaller mean ΔHR 1min (23 beats per minute [bpm], 95% CI 20-26 vs 32 bpm, 95% CI 26-37, P = .006) and the difference between maximal HR and HR at 2 minutes (39 bpm, 95% CI 36-43 vs 48 bpm, 95% CI 42-53, P = .011). Subjects with SCA also showed smaller mean changes in HR from peak HR to 1 minute, from 1 minute to 2 minutes, and from 2 through 5 minutes of recovery by repeated-measures testing. In a multivariable regression model, older age was independently associated with smaller ΔHR 1min in subjects with SCA. Cardiopulmonary fitness and hydroxyurea use, however, were not independent predictors of ΔHR 1min . Conclusions Children with SCA demonstrate impaired HRR after maximal exercise. Reduced postexercise HRR in SCA suggests impaired parasympathetic function, which may become progressively worse with age, in this population.

Background Although anthracycline therapy is invaluable for treating neoplastic disorders, morbidity includes severe cardiomyopathy that leads to heart transplantation. This multicenter study describes the course of children who experienced anthracycline cardiomyopathy (ACM) and who subsequently required heart transplantation. Methods We reviewed transplant databases/registries at 4 pediatric heart transplant centers to identify children with ACM who were listed for heart transplantation. We reviewed medical records to determine cancer therapy, clinical course, and outcome. Results Eighteen patients were listed, and 17 underwent transplantation. Mean age at cancer diagnosis was 6.0 years (SD, 3.7). The mean anthracycline dose was 361 mg/m2 (SD, 110). The median time from cancer diagnosis to listing for heart transplantation was 9.2 years (range, 0.4–15.2 years). Six transplantations were performed in patients who had disease-free intervals of

Abstract 3220 Slow heart rate recovery (HRR) after exercise, particularly at 1 and 2 minutes during recovery, is a strong indicator of autonomic nervous system (ANS) imbalance and represents an important risk factor for increased cardiovascular morbidity and mortality in the general population. Recent evidence suggests cardiovascular ANS dysfunction and vagal tone impairment also exist in individuals with sickle cell anemia (SCA). Despite the known impact of disease burden on exercise capacity and overall fitness in SCA, post-exercise HRR has not previously been studied in this population. The objective of this study was to examine HRR in the post-exercise recovery phase following maximal exercise testing in a cohort of children and young adults with SCA. Methods We prospectively performed maximal cardiopulmonary exercise testing using a ramp, cycle ergometry protocol in 60 subjects with SCA (hemoglobin SS and S-β0 thalassemia) and 20 controls without SCA or sickle cell trait matched for race and gender. Data from standard 12-lead electrocardiography (ECG) was assessed during a 10-minute recovery phase, and HR and corrected QT (QTc) interval measured from tracings obtained at 1-minute intervals. A single investigator performed all measurements manually using leads II, V5, and V6. Final values were averaged from 3 independent measurements. The difference between maximal HR and both HR at 1-minute (ΔHR1min) and 2-minute (ΔHR2min) recovery was our primary outcome. Between-group differences were compared using Mann-Whitney U testing (IBM, SPSS V20). The relationship between ΔHR1min and exercise parameters was examined using Spearman's rank correlation coefficient. Results Post-exercise ECG data from 58 subjects with SCA (median age 15.5 years) and 20 controls without SCA (median age 12 years) were interpretable for this analysis. A total of 30/58 (52%) and 22/58 (38%) subjects were male and on hydroxyurea, respectively. There was no significant difference in median baseline HR or QTc interval in subjects versus controls. Median peak HR was also not significantly different in the 2 groups. When compared to controls, subjects with SCA demonstrated significantly lower HR reserve (100 vs. 111 bpm, p = 0.005), representing the difference between peak and baseline HR. Impaired HRR, defined by slower declines in HR during recovery, was also evident among subjects with SCA following maximal exercise challenge. Although the absolute HR measured at each minute of recovery did not differ significantly in the 2 groups, subjects demonstrated significantly smaller median ΔHR1min (21 vs. 33 bpm, p < 0.0001) and ΔHR2min (39 vs. 51 bpm, p = 0.003), even after adjustment for age between groups. Significantly smaller ΔHR values were also noted in subjects at each minute up to 8 minutes throughout recovery. When compared to subjects not on hydroxyurea, subjects on treatment had significantly greater median ΔHR1min (25 vs. 19 bpm, p = 0.037) but similar ΔHR2min. In subjects with SCA, ΔHR1min correlated inversely with age (−0.41, p = 0.001) and directly with peak oxygen consumption (peak VO2) (0.30, p = 0.03) and oxygen delivery (ΔVO2/Δwork rate) (0.46, p < 0.0001), but not with baseline QTc interval or hemoglobin. Through mono-exponential curve fitting, we found that the time constant associated with HR decline over the first 5 minutes of recovery was greater in subjects with SCA versus controls (T = 128 sec, 95% CI [123, 134] versus 104 sec, 95% [97, 110]). Conclusions Compared to controls without SCA, children and young adults with SCA demonstrate impaired HRR following maximal cardiopulmonary exercise testing, as evidenced by smaller decrements in HR at 1 and 2 minutes during recovery and exponential time constants that indicate a longer period of time over which HR declines. Slower HRR is also associated with decreased fitness and measures of oxygen delivery during exercise testing in subjects with SCA. Although the mechanisms are not well understood, prolonged HRR in this population may in part be explained by ANS imbalance, suggesting either inadequate sympathetic withdrawal or suboptimal parasympathetic reactivation in the post-exercise period. Future studies should focus on delineating the role of the ANS in post-exercise HRR and assessing the prognostic potential of this marker as it relates to disease severity and outcomes in SCA. Disclosures: No relevant conflicts of interest to declare.

All had at least 2 determinations for postTx alloantibodies. Overall 229 samples were analyzed. Results: Twenty-two of 45 had preand/or postTx antiHLA-ab: 77% in A (17/22) and only 22% in B (5/23), p 0.0002. PreTx HLA-ab were present in 12 cases (27%): 8 class I II and 4 class I. Presensitization was more frequent in A (11/ 22 50%) than in B (1/ 23 4%, p 0.0005). Thirteen cases retained (8 cases) or developed (5 cases) antiHLA-ab during the 1st year postTx: 10 in A (50%) and 3 in B (13%), p 0.0008. Late after Tx ( 1year), antiHLA-ab were present in 8/18 (44%) in A and 3/22 (13.6%) in B (p 0.03). Eleven exhibited de novo antibodies at any time postTx (6 class I II, 4 class I and 1 class II): 7/22 in A (32%) and 4/23 in B (17%, p 0.0004). Four of 8 cases with CAD (50%) had preformed antiHLA-ab (3 retained postTx) compared to 8 of 37 without CAD (25.6%), small sample size for statistical analysis (p 0.09); none of CAD cases exhibited de novo antibodies. Conclusion: Preformed, persistent and de novo ELISA-detected antiHLA-ab were correlated with first-year acute rejection profile; further investigations with larger number of patients are needed to determine correlation with graft coronary disease.

Objectives We assessed our pacemaker strategy, use of antitachycardia therapies, generator longevity, and need for programming changes in patients having Fontan conversion with arrhythmia surgery. Methods Between 1994 and 2008, of 121 consecutive patients having Fontan conversion and arrhythmia surgeries, 120 patients underwent pacemaker implantation at the time of Fontan conversion (mean age 22.9 ± 8.1 years). Prior pacemakers were in place in 32/120 (26.7%) patients. Between 1994 and 1998, single-chamber atrial antitachycardia pacemakers were implanted (n = 12); atrial rate-responsive pacemakers (n = 31) were implanted between 1998 and 2002. Dual-chamber rate-responsive pacemakers (n = 16) were used between 2002 and 2003, and subsequently dual-chamber antitachycardia pacemakers (n = 61) have become the pacemaker of choice. Leads have evolved from transatrial endocardial leads to epicardial unipolar and subsequently bipolar leads. Results Among 87 patients with adequate follow-up, all are currently atrially paced at a minimum lower rate ≥70 beats per minute. Single-chamber atrial pacemakers were implanted in 43 (antitachycardia in 12), and dual-chamber pacemakers in 77 (antitachycardia in 61); multisite ventricular leads were placed in 7 patients. Far-field R-wave sensing in 78.6% of unipolar atrial leads led to use of epicardial bipolar leads. Late atrioventricular block (24%) led to routine implantation of dual-chamber pacemakers. Antitachycardia pacing was utilized in 7%. One patient required acute lead revision and 4 required late upgrade to dual-chamber pacemakers. There was no pacemaker-related infection. Twenty patients required generator change, and the mean device longevity was 7.53 years. Conclusions Customized pacemaker therapy can optimize management of patients following Fontan conversion. Device longevity is excellent. Based on our experience with 120 Fontan conversions, we recommend placement of a dual-chamber antitachycardia pacemaker with bipolar steroid-eluting epicardial leads in all patients at the time of the conversion.